关键词: Aggregatibacter actinomycetemcomitans Cathepsin C Oral metagenome Papillon-Lefèvre-syndrome Periodontitis Periodontopathogens Recessive mutation

Mesh : Humans Papillon-Lefevre Disease Periodontitis / therapy Health Status

来  源:   DOI:10.1186/s12903-024-03856-z   PDF(Pubmed)

Abstract:
OBJECTIVE: The oral microbiota composition of patients diagnosed with Papillon-Lefèvre-syndrome and treated for several years were compared to those existing in the oral cavity of the clinically healthy family members and a cohort of patients having various stages of chronic periodontitis.
METHODS: A family with two sisters affected with severe periodontitis and with the typical skin symptoms of Papillon-Lefèvre-syndrome, and symptomless parents and third sibling were investigated. The Patients received periodontal treatment for several years and their oral microbiome was analysed by amplicon sequencing. Data were evaluated by microbial cluster analysis.
RESULTS: The microbiome of the patients with Papillon-Lefèvre-syndrome was predominated with Aggregatibacter actinomycetemcomitans and associated oral periodontopathogens. Although the clinically healthy family members showed no oral disorder, their microbiome resembled that of subjects having mild periodontitis.
CONCLUSIONS: Predominance of A. actinomycetemcomitans in the subgingival microbiome of patients with Papillon-Lefèvre-syndrome suggests that specific treatment strategies directed against this pathobiont may improve the oral health status of the affected individuals.
BACKGROUND: The study was conducted in accordance with the Declaration of Helsinki and the ethical permission has been issued by the Human Investigation Review Board of the University of Szeged, Albert Szent-Györgyi Clinical Centre (Permission No. 63/2017-SZTE). September 19, 2017.  https://u-szeged.hu/klinikaikutatas/rkeb-altal-jovahagyott/rkeb-2017 .
摘要:
目的:将诊断为Papillon-Lefèvre综合征并治疗数年的患者的口腔微生物组成与临床健康的家庭成员和患有不同阶段的慢性牙周炎患者的口腔中存在的微生物组成进行比较。
方法:一个有两个姐妹的家庭患有严重牙周炎,并有典型的Papillon-Lefèvre综合征的皮肤症状,并对无症状的父母和第三个兄弟姐妹进行了调查。患者接受牙周治疗数年,并通过扩增子测序分析其口腔微生物组。通过微生物聚类分析评估数据。
结果:Papillon-Lefèvre综合征患者的微生物群以放线菌和相关的口腔牙周病菌为主。尽管临床健康的家庭成员没有表现出口腔疾病,他们的微生物组类似于患有轻度牙周炎的受试者。
结论:Papillon-Lefèvre综合征患者的龈下微生物群中放线菌的优势表明,针对这种疾病的特定治疗策略可能会改善受影响个体的口腔健康状况。
背景:这项研究是根据《赫尔辛基宣言》进行的,并且塞格德大学人类调查审查委员会已发布道德许可,AlbertSzent-Györgyi临床中心(许可号63/2017-SZTE)。2017年9月19日。https://u-szeged.hu/klinikaikutatas/rkeb-altal-jovahagyott/rkeb-2017.
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